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Related Concept Videos

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Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
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As muscle contracts, the overlap between the thin and thick filaments increases, decreasing the length of the sarcomere—the contractile unit of the muscle—using energy in the form of ATP. At the molecular level, this is a cyclic, multistep process that involves binding and hydrolysis of ATP, and movement of actin by myosin.
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Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
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Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
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Related Experiment Video

Updated: Sep 5, 2025

Tissue Triage and Freezing for Models of Skeletal Muscle Disease
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Skeletal Muscle Pathogenesis in Polyglutamine Diseases.

Caterina Marchioretti1,2, Emanuela Zuccaro1,2, Udai Bhan Pandey3

  • 1Department of Biomedical Sciences (DBS), University of Padova, 35131 Padova, Italy.

Cells
|July 9, 2022
PubMed
Summary

Polyglutamine diseases impact skeletal muscles, contributing to disease progression. This review covers skeletal muscle

Keywords:
Huntington’s diseasepolyglutamine diseasesskeletal muscle atrophyspinal and bulbar muscular atrophyspinocerebellar ataxia

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Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Polyglutamine diseases involve selective neuronal degeneration in the central nervous system.
  • Nonneuronal cells, including skeletal muscle, can be affected in these diseases.
  • Skeletal muscle atrophy is a significant factor in polyglutamine disease progression.

Purpose of the Study:

  • To review the impact and relevance of skeletal muscle in polyglutamine diseases.
  • To discuss evidence from animal models and patient-derived cells regarding skeletal muscle involvement.

Main Methods:

  • Review of existing literature on polyglutamine diseases and skeletal muscle pathology.
  • Analysis of data from animal models recapitulating skeletal muscle atrophy.
  • Examination of findings from patient-derived cell models.

Main Results:

  • Skeletal muscle is a primary site of toxicity for certain polyglutamine-expanded proteins.
  • Skeletal muscle atrophy significantly impacts whole-body metabolism, worsening disease.
  • Animal models and cell studies effectively model skeletal muscle pathology in these diseases.

Conclusions:

  • Skeletal muscle plays a critical role in the pathogenesis and progression of polyglutamine diseases.
  • Understanding skeletal muscle involvement is crucial for developing effective therapeutic strategies.